Open Access

ARTICLE

Health-Related Quality of Life, Emotional and Behavioral Problems in Children and Adolescents with Ebstein Anomaly

by Lianne M. Geerdink^1,2,*, Malindi van der Mheen^3,4, Gideon J. du Marchie Sarvaas⁵, Irene M. Kuipers⁶, Stefan Frerich⁷, Henriëtte ter Heide², Willem A. Helbing⁸, Zina Feijzic¹, Christian Schroer⁹, Chris L. de Korte¹⁰, Livia Kapusta^1,11, Chris M. Verhaak¹², Elisabeth M. W. J. Utens^3,4

1 Department of Pediatric Cardiology, Amalia Children’s Hospital, University Medical Center Nijmegen, Nijmegen, 6525 GA, The Netherlands
2 Department of Pediatric Cardiology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, 3584 EA, The Netherlands
3 Academic Center for Child and Adolescent Psychiatry the Bascule/Department of Child and Adolescent Psychiatry, Amsterdam University Medical Center, Amsterdam, 1105 AZ, The Netherlands
4 Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Center, Rotterdam, 3015 GD, The Netherlands
5 Center for Congenital Heart Diseases, University Medical Center Groningen, Groningen, 9713 GZ, The Netherlands
6 Department of Pediatric Cardiology, Academic Medical Center Amsterdam, Amsterdam, 1105 AZ, The Netherlands
7 Department of Pediatric Cardiology, Maastricht University Medical Center, Maastricht, 6229 HX, The Netherlands
8 Department of Pediatric Cardiology, Sophia Children’s Hospital, Erasmus Medical Center, Rotterdam, 3015 GD, The Netherlands
9 Department of Pediatrics, Máxima Medical Center Veldhoven, Veldhoven, 5504 DB, The Netherlands
10 Department of Radiology, Medical Ultrasound Imaging Center, University Medical Center Nijmegen, Nijmegen, 6525 GA, The Netherlands
11 Pediatric Cardiology Unit, Department of Pediatrics, Dana-Dwek Children’s Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, 6423906, Israel
12 Department of Medical Psychology, Amalia Children’s Hospital, University Medical Center Nijmegen, Nijmegen, 6525 GA, The Netherlands

* Corresponding Author: Lianne M. Geerdink. Email:

Congenital Heart Disease 2020, 15(6), 457-472. https://doi.org/10.32604/CHD.2020.012994

Received 21 July 2020; Accepted 22 September 2020; Issue published 02 November 2020

Abstract

Background: Due to the improved survival rates of children and adolescents with congenital heart disease (CHD), more attention is now being directed towards their health-related quality of life (HRQoL), emotional and behavioral problems. Ebstein anomaly (EA) is a rare CHD with a broad clinical spectrum. The aim of the current study is to evaluate self- and proxy-reported HRQoL and emotional and behavioral problems in children and adolescents with EA. Methods: In this cross-sectional, multicenter study, we included EA patients (aged 8–17 years), who underwent routine clinical assessments in Dutch university hospitals between May 2017 and March 2019. The Generic Pediatric Quality of Life Inventory 4.0 (for ages 8–12/13–17 years) was used to assess HRQoL. The Child Behavior Checklist, Youth Self-Report and Teacher’s Report Form were completed to measure emotional and behavioral problems. Results: Questionnaires for thirty-eight patients (median age: 13 years; 48% male) were completed by patients themselves, parents and teachers. Compared to normative data, self-reported HRQoL was not significantly impaired, except for social functioning in children aged 8–12 years. Gender, severity of tricuspid regurgitation or previous surgery did not predict HRQoL. Parents reported significantly more internalizing (anxiety/depression), attention and thought problems in children with EA. These problems, however, were not reported by adolescents themselves. School teachers reported significantly fewer externalizing problems compared to the norm. Conclusions: Overall, despite satisfactory HRQoL, children and adolescents show emotional and behavioral problems. We recommend routine HRQoL and psychological assessment to screen for less apparent internalizing and psychosocial problems.

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